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Muenke Syndrome

MORPHOLOGICAL CHARACTERISTICS

The clinical picture of Muenke syndrome is usually similar to the picture of unilateral or bilateral coronal suture craniosynostosis. The difference is that in the case of Muenke syndrome there is an obvious genetic diagnosis and inheritance (in contrast with non-syndromic types of coronal craniosynostosis).

Muenke syndrome. Severe right plagiocephaly.View from the top of the headPostoperative appearancePostoperative view from the top of the headThe father of the child. Brachycephalic skull structureWide forehead, typical of brachycephalyThe child's grandmother. Plagiocephalic cranial structure (mild)Head hyperextended which emphasizes the plagiocephaly

 

 

Muenke syndrome. Three-dimensional imaging.View from the top of the headPostoperative three-dimensional imagingPostoperative view from the top of the head. The bone defects result from the movement of the bones and the growth of the skull and are insignificantMagnetic resonance imaging. Asymmetry of the cerebral hemispheres

Other morphological abnormalities that have been observed in Muenke syndrome cases are the following:

  • Vaulted palate that may predispose to serous otitis
  • cleft lip or/and palate (rare)

The clinical diagnosis of the syndrome requires clinical experience as well as a high index of suspicion and it is confirmed with genetic testing.

SURGICAL REHABILITATION

Unless there are specific characteristics, the surgical repair is the same as the frontal plagiocephaly repair (see relevant chapter). It is estimated that 20-25% of the cases that have been diagnosed with Muenke syndrome will need reoperation because the malformation is often profound and its full repair is very difficult to be achieved by the initial surgery.

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